Connective tissue disease-associated pulmonary arterial hypertension in the modern treatment era.
BACKGROUND
Pulmonary arterial hypertension in association with connective tissue disease (CTD-PAH) has historically had a poor prognosis, with a 1-year survival rate among patients with systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) of 45%. However, more therapies have become available.


OBJECTIVE
To investigate the survival and characteristics of all patients diagnosed with CTD-PAH in the U.K. pulmonary hypertension service.


METHODS
National registry of all incident cases of CTD-PAH diagnosed consecutively between January 2001 and June 2006.


RESULTS
Patients with CTD-PAH (429; 73% SSc-PAH) were diagnosed by a catheter-based approach. One- and 3-year survival rates were 78 and 47% for patients with isolated SSc-PAH. Survival was worse for those with respiratory disease-associated SSc-PAH (3-yr survival, 28%; P = 0.005) whereas survival among patients with exercise-induced SSc-PAH was superior (3-yr survival, 86%; P = < 0.001). Age, sex, mixed venous oxygen saturation, and World Health Organization functional class were independent predictors of survival in isolated SSc-PAH. Nineteen percent of patients with exercise-induced SSc-PAH and 39% of patients with isolated SSc-PAH who were in functional classes I and II had evidence of disease progression. The prevalence of diagnosed SSc-PAH is 2.93 per 1 million. The 3-year survival rate of 75% for those with pulmonary arterial hypertension associated with systemic lupus erythematosus (SLE-PAH) was significantly better than that for patients with SSc-PAH (P = 0.01).


CONCLUSIONS
Survival of patients with SSc-PAH in the modern treatment era is better than in historical series. A significant proportion of patients with mild functional impairment or exercise-induced SSc-PAH have evidence of disease progression. Survival of patients with respiratory disease-associated pulmonary hypertension is inferior. SLE-PAH has a better prognosis than SSc-PAH.
Condliffe
Robin
R
Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield, United Kingdom.
Kiely
David G
DG
Peacock
Andrew J
AJ
Corris
Paul A
PA
Gibbs
J Simon R
JS
Vrapi
Florenc
F
Das
Clare
C
Elliot
Charlie A
CA
Johnson
Martin
M
DeSoyza
Julia
J
Torpy
Chantal
C
Goldsmith
Kim
K
Hodgkins
Denise
D
Hughes
Rodney J
RJ
Pepke-Zaba
Joanna
J
Coghlan
J Gerry
JG
D000368:Aged; D015331:Cohort Studies; D003240:Connective Tissue Diseases / Q000150:complications* / Q000175:diagnosis; D005081:Exercise Therapy; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000209:etiology / Q000401:mortality* / Q000628:therapy*; D008180:Lupus Erythematosus, Systemic / Q000150:complications; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012307:Risk Factors; D012595:Scleroderma, Systemic / Q000150:complications; D015996:Survival Rate; D006113:United Kingdom / Q000453:epidemiology